Skip to main content
PMC home page
Postgraduate Medical Journal logoLink to Postgraduate Medical Journal
. 1991 Jul;67(789):632–637. doi: 10.1136/pgmj.67.789.632

Lung function abnormalities and decline of spirometry in scleroderma: an overrated danger?

M J Abramson 1, A J Barnett 1, G O Littlejohn 1, M M Smith 1, S Hall 1
PMCID: PMC2399092  PMID: 1924047

Abstract

To document the prevalence and progression of pulmonary involvement in scleroderma (systemic sclerosis including the CREST syndrome), the clinical notes and lung function records of 113 cases were reviewed. Lung function was normal in 39 cases, isolated impairment of DLCO was found in 38 patients, a restrictive defect was present in 27 cases and there was evidence of airflow obstruction in 9 cases. The median duration of symptoms was 10 years. Dyspnoea and an interstitial pattern on chest X-ray were associated with impaired lung function. Death during the period of review was significantly related to initial impairment of the DLCO. Sixty-six patients (53 women and 13 men) underwent repeat spirometry at least 1 year after initial testing. The rates of change in VC and FEV1 were no more rapid than would be expected for normal subjects. There was no significant difference in rates of change between men and women or between dyspnoeic patients and those who were asymptomatic. The extent of skin involvement and the presence of interstitial fibrosis on chest X-ray were unrelated to the rate of loss of lung function. It is concluded that most scleroderma patients in this study had abnormal lung function when first tested, but overall significant worsening of spirometry was not found.

Full text

PDF

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. ATS statement--Snowbird workshop on standardization of spirometry. Am Rev Respir Dis. 1979 May;119(5):831–838. doi: 10.1164/arrd.1979.119.5.831. [DOI] [PubMed] [Google Scholar]
  2. Bagg L. R., Hughes D. T. Serial pulmonary function tests in progressive systemic sclerosis. Thorax. 1979 Apr;34(2):224–228. doi: 10.1136/thx.34.2.224. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Barnett A. J., Miller M. H., Littlejohn G. O. A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol. 1988 Feb;15(2):276–283. [PubMed] [Google Scholar]
  4. Burrows B., Lebowitz M. D., Camilli A. E., Knudson R. J. Longitudinal changes in forced expiratory volume in one second in adults. Methodologic considerations and findings in healthy nonsmokers. Am Rev Respir Dis. 1986 Jun;133(6):974–980. doi: 10.1164/arrd.1986.133.6.974. [DOI] [PubMed] [Google Scholar]
  5. Colp C. R., Riker J., Williams M. H., Jr Serial changes in scleroderma and idiopathic interstitial lung disease. Arch Intern Med. 1973 Oct;132(4):506–515. [PubMed] [Google Scholar]
  6. Eason R. J., Tan P. L., Gow P. J. Progressive systemic sclerosis in Auckland: a ten year review with emphasis on prognostic features. Aust N Z J Med. 1981 Dec;11(6):657–662. doi: 10.1111/j.1445-5994.1981.tb03542.x. [DOI] [PubMed] [Google Scholar]
  7. Greenwald G. I., Tashkin D. P., Gong H., Simmons M., Duann S., Furst D. E., Clements P. Longitudinal changes in lung function and respiratory symptoms in progressive systemic sclerosis. Prospective study. Am J Med. 1987 Jul;83(1):83–92. doi: 10.1016/0002-9343(87)90501-8. [DOI] [PubMed] [Google Scholar]
  8. Medsger T. A., Jr D-penicillamine treatment of lung involvement in patients with systemic sclerosis (scleroderma). Arthritis Rheum. 1987 Jul;30(7):832–834. doi: 10.1002/art.1780300716. [DOI] [PubMed] [Google Scholar]
  9. Medsger T. A., Jr, Masi A. T., Rodnan G. P., Benedek T. G., Robinson H. Survival with systemic sclerosis (scleroderma). A life-table analysis of clinical and demographic factors in 309 patients. Ann Intern Med. 1971 Sep;75(3):369–376. doi: 10.7326/0003-4819-75-3-369. [DOI] [PubMed] [Google Scholar]
  10. Miller M. H., Littlejohn G. O., Davidson A., Jones B., Topliss D. J. The clinical significance of the anticentromere antibody. Br J Rheumatol. 1987 Feb;26(1):17–21. doi: 10.1093/rheumatology/26.1.17. [DOI] [PubMed] [Google Scholar]
  11. Owens G. R., Follansbee W. P. Cardiopulmonary manifestations of systemic sclerosis. Chest. 1987 Jan;91(1):118–127. doi: 10.1378/chest.91.1.118. [DOI] [PubMed] [Google Scholar]
  12. Peters-Golden M., Wise R. A., Hochberg M. C., Stevens M. B., Wigley F. M. Carbon monoxide diffusing capacity as predictor of outcome in systemic sclerosis. Am J Med. 1984 Dec;77(6):1027–1034. doi: 10.1016/0002-9343(84)90183-9. [DOI] [PubMed] [Google Scholar]
  13. Peters-Golden M., Wise R. A., Schneider P., Hochberg M., Stevens M. B., Wigley F. Clinical and demographic predictors of loss of pulmonary function in systemic sclerosis. Medicine (Baltimore) 1984 Jul;63(4):221–231. doi: 10.1097/00005792-198407000-00004. [DOI] [PubMed] [Google Scholar]
  14. RITCHIE B. PULMONARY FUNCTION IN SCLERODERMA. Thorax. 1964 Jan;19:28–36. doi: 10.1136/thx.19.1.28. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Schneider P. D., Wise R. A., Hochberg M. C., Wigley F. M. Serial pulmonary function in systemic sclerosis. Am J Med. 1982 Sep;73(3):385–394. doi: 10.1016/0002-9343(82)90732-x. [DOI] [PubMed] [Google Scholar]
  16. Steen V. D., Owens G. R., Fino G. J., Rodnan G. P., Medsger T. A., Jr Pulmonary involvement in systemic sclerosis (scleroderma). Arthritis Rheum. 1985 Jul;28(7):759–767. doi: 10.1002/art.1780280706. [DOI] [PubMed] [Google Scholar]
  17. Steen V. D., Owens G. R., Redmond C., Rodnan G. P., Medsger T. A., Jr The effect of D-penicillamine on pulmonary findings in systemic sclerosis. Arthritis Rheum. 1985 Aug;28(8):882–888. doi: 10.1002/art.1780280807. [DOI] [PubMed] [Google Scholar]
  18. Ungerer R. G., Tashkin D. P., Furst D., Clements P. J., Gong H., Jr, Bein M., Smith J. W., Roberts N., Cabeen W. Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis. Am J Med. 1983 Jul;75(1):65–74. doi: 10.1016/0002-9343(83)91169-5. [DOI] [PubMed] [Google Scholar]

Articles from Postgraduate Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES